Davidenkow's syndrome : a clinical, electrophysiological and histomorphometric evaluation

Davidenkow's syndrome is being reported in three members of a family. The affected subjects had weakness and wasting of the deltoids and the distal muscles of the lower limbs. The onset of the disorder was in the middle of the second decade and the affected subjects retained the capability of independent mobility till the fifth decade. Neuroelectromyographic studies revealed the presence of a demyelinating neuropathy. Sural nerve histology was characterised by loss of myelinated fibres and segmental demyelination.